Endoscopic endonasal odontoidectomy in a patient affected by Down syndrome: technical case report.

Department of Neurosurgery, Surgery Center for Pituitary Tumors, Bellaria Hospital, Bologna, Italy.

OBJECTIVE: Atlantoaxial instability and odontoid invagination may occur in Down syndrome. The treatment of this lesion may require an odontoidectomy. In similar circumstances, a transoral procedure may be hampered by a macroglossia which is part of Down syndrome. In a situation occurring in an 11-year-old child affected by atloaxial instability and os odontoideum, the persistence of ventral compression after a previous atloaxial fixation induced us to perform an endoscopic endonasal odontoidectomy. We feel that this is an elective indication for endoscopic endonasal odontoidectomy. CLINICAL PRESENTATION: An 11-year-old child, affected by Down syndrome, presented with progressive tetraparesis with severe superior diplegia. He was wheelchair-bound. Recently, respiratory disturbances had appeared. INTERVENTION: The patient underwent an endoscopic endonasal odontoidectomy. The postoperative course was uneventful, and the patient experienced improvement of the preoperative symptoms. CONCLUSION: An endonasal technique is indicated for patients presenting with narrowness of the oral cavity, such as in the case reported, where the narrowness due to pediatric age and the macroglossia characteristic of Down syndrome hamper the transoral approach.

PMID: 18797319 [PubMed - indexed for MEDLINE]